BackgroundBiliary tract cancer (BTC) is a rare malignant neoplasm with poor prognosis. We aimed to report the rates and the trends of biliary tract cancer at the global level from 1990 to 2017 according to gender, age and social-demographic index (SDI). MethodsData on biliary tract cancer between 1990 and 2017 was acquired from the 2017 Global Burden of Disease (GBD) study including the incidence, death and disability adjusted life-years (DALY). We also calculated estimated annual percentage changes (EAPC) to quantificationally describe the trend of incidence, death and DALY over time. ResultsOverall, the number of biliary tract cancer increased from 119,943 in 1990 to 210,878 in 2017. The age-standardized incidence rate (ASIR) decreased continuously in this period with an EAPC of -0.56 (−0.67 to −0.45). The age-standardized death rate (ASDR) declined from 2.79 (95 % CI 2.68 − 3.14) in 1990 to 2.23 (95 % CI 1.99 − 2.38) per 100,000 persons in 2017. In terms of SDI, ASIR and ASDR were increased in low- and low-middle- SDI regions, but decreased in the other three regions, so was the age-standardized DALY rate. The ASR (Age-standardized incidence/mortality/DALY rate) varied across the world, with the highest ASIR and ASDR in Chile, and the lowest ones in Iraq. ConclusionThe ASIR, ASDR and age-standardized DALY rate of biliary tract cancer have decreased at the global level. However, changes in rates vary among different counties and regions, suggesting it is more reasonable to adopt targeted and specific measures to decrease the occurrence of cancer.